KEGG   ENZYME: 4.1.2.63
Entry
EC 4.1.2.63                 Enzyme                                 
Name
2-hydroxyacyl-CoA lyase;
HACL1 (gene name);
2-hydroxyphytanoyl-CoA lyase;
2-HPCL
Class
Lyases;
Carbon-carbon lyases;
Aldehyde-lyases
Sysname
2-hydroxy-3-methyl fatty-CoA formyl-CoA lyase (2-methyl branched fatty aldehyde-forming)
Reaction(IUBMB)
(1) a 2-hydroxy-3-methyl-Cn-fatty-acyl-CoA = a 2-methyl-branched Cn-1-fatty aldehyde + formyl-CoA [RN:R12799];
(2) a (2R)-2-hydroxy-Cn-long-chain fatty acyl-CoA = a Cn-1-long-chain fatty aldehyde + formyl-CoA [RN:R12800]
Reaction(KEGG)
R12799 R12800
Substrate
2-hydroxy-3-methyl-Cn-fatty-acyl-CoA;
(2R)-2-hydroxy-Cn-long-chain fatty acyl-CoA
Product
2-methyl-branched Cn-1-fatty aldehyde;
formyl-CoA [CPD:C00798];
Cn-1-long-chain fatty aldehyde
Comment
Requires Mg2+ and thiamine diphosphate. This peroxisomal enzyme, found in animals, is involved in the alpha-oxidation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids.
History
EC 4.1.2.63 created 2021
Orthology
K12261  2-hydroxyacyl-CoA lyase
Genes
HSA: 26061(HACL1)
PTR: 743738(HACL1)
PPS: 100982158(HACL1)
GGO: 101130205(HACL1)
PON: 100457302(HACL1)
NLE: 100589385(HACL1)
MCC: 694261(HACL1)
MCF: 102130550(HACL1)
CSAB: 103241708(HACL1)
CATY: 105578325(HACL1)
PANU: 101016987(HACL1)
TGE: 112617609(HACL1)
RRO: 104656450(HACL1)
RBB: 108535627(HACL1)
TFN: 117084204(HACL1)
PTEH: 111539503(HACL1)
CJC: 100385052(HACL1)
SBQ: 101042137(HACL1)
CSYR: 103249270(HACL1)
MMUR: 105872704(HACL1)
OGA: 100942962(HACL1)
MMU: 56794(Hacl1)
MCAL: 110309041(Hacl1)
MPAH: 110325771(Hacl1)
RNO: 85255(Hacl1)
MCOC: 116084402(Hacl1)
MUN: 110551269(Hacl1)
CGE: 100767514(Hacl1)
PLEU: 114708165(Hacl1)
NGI: 103747520(Hacl1)
HGL: 101706829(Hacl1)
CPOC: 100731806(Hacl1)
CCAN: 109694810(Hacl1)
DORD: 105989902(Hacl1)
DSP: 122107948(Hacl1)
OCU: 100342558(HACL1)
OPI: 101524332(HACL1)
TUP: 102488706(HACL1)
CFA: 608697
VVP: 112925619(HACL1)
VLG: 121478711(HACL1)
AML: 100483076(HACL1)
UMR: 103679362(HACL1)
UAH: 113253912(HACL1)
UAR: 123788357(HACL1)
ELK: 111155096
LLV: 125104689
MPUF: 101679744(HACL1)
ORO: 101380715(HACL1)
EJU: 114205724(HACL1)
ZCA: 113915823(HACL1)
MLX: 118003367(HACL1)
FCA: 101093708(HACL1)
PYU: 121033647(HACL1)
PBG: 122492011(HACL1)
PTG: 102951290(HACL1)
PPAD: 109258811(HACL1)
AJU: 106971590(HACL1)
HHV: 120238469(HACL1)
BTA: 512676(HACL1)
BOM: 102283180(HACL1)
BIU: 109561790(HACL1)
BBUB: 102399404(HACL1)
CHX: 102182450(HACL1)
OAS: 101119009(HACL1)
ODA: 120880360(HACL1)
CCAD: 122445507(HACL1)
SSC: 100518218(HACL1)
CFR: 102508961(HACL1)
CBAI: 105077623(HACL1)
CDK: 105096613(HACL1)
VPC: 102525121(HACL1)
BACU: 103005299(HACL1)
LVE: 103081479(HACL1)
OOR: 101278567(HACL1)
DLE: 111168157(HACL1)
PCAD: 102980162(HACL1)
PSIU: 116753296(HACL1)
ECB: 100063920(HACL1)
EPZ: 103561485(HACL1)
EAI: 106842763(HACL1)
MYB: 102259094(HACL1)
MYD: 102760568(HACL1)
MMYO: 118668201(HACL1)
MLF: 102419042(HACL1)
MNA: 107542410(HACL1)
PKL: 118715364(HACL1)
HAI: 109395823(HACL1)
DRO: 112309688(HACL1)
SHON: 118997591(HACL1)
AJM: 119048308(HACL1)
PDIC: 114501580(HACL1)
PHAS: 123830654(HACL1)
MMF: 118633385(HACL1)
RFQ: 117037187(HACL1)
PALE: 102884140(HACL1)
PGIG: 120582055(HACL1)
PVP: 105288363(HACL1)
RAY: 107508452(HACL1)
MJV: 108390849(HACL1)
TOD: 119256849(HACL1)
SARA: 101543974(HACL1)
LAV: 100667352(HACL1)
TMU: 101354112
DNM: 101437479(HACL1)
MDO: 100030500(HACL1)
GAS: 123250584(HACL1)
SHR: 100916928(HACL1)
PCW: 110209056(HACL1)
OAA: 100084023(HACL1)
GGA: 420638(HACL1)
PCOC: 116227085(HACL1)
MGP: 100542074(HACL1)
CJO: 107309164(HACL1)
NMEL: 110394950(HACL1)
APLA: 101798319(HACL1)
ACYG: 106030248(HACL1)
AFUL: 116486217(HACL1)
TGU: 100232217(HACL1)
LSR: 110474620(HACL1)
SCAN: 103812914(HACL1)
PMOA: 120502665(HACL1)
OTC: 121339586(HACL1)
PRUF: 121352912(HACL1)
GFR: 102037543(HACL1)
FAB: 101821112(HACL1)
PHI: 102104383(HACL1)
PMAJ: 107199916(HACL1)
CCAE: 111923442(HACL1)
CCW: 104689075(HACL1)
ETL: 114055681(HACL1)
ZAB: 102070040(HACL1)
FPG: 101915302(HACL1)
FCH: 102057939(HACL1)
CLV: 102085572(HACL1)
EGZ: 104126084(HACL1)
NNI: 104013970(HACL1)
ACUN: 113476794(HACL1)
TALA: 104358157(HACL1)
PADL: 103918430(HACL1)
ACHC: 115339770(HACL1)
AAM: 106483474(HACL1)
AROW: 112977277(HACL1)
NPD: 112957212(HACL1)
DNE: 112985274(HACL1)
ASN: 102372627(HACL1)
AMJ: 102569780(HACL1)
CPOO: 109310399(HACL1)
GGN: 109291599(HACL1)
PSS: 102457478(HACL1)
CMY: 102946745(HACL1)
CPIC: 101939557(HACL1)
TST: 117872359(HACL1)
CABI: 116837022(HACL1)
MRV: 120398934(HACL1)
ACS: 100557174(hacl1)
PVT: 110072147(HACL1)
SUND: 121934086(HACL1)
PBI: 103048759(HACL1)
PMUR: 107282910(HACL1)
TSR: 106545060(HACL1)
PGUT: 117655106(HACL1)
VKO: 123033833(HACL1)
PMUA: 114607097(HACL1)
ZVI: 118092350(HACL1)
GJA: 107126170(HACL1)
XLA: 446657(hacl1.L)
XTR: 550086(hacl1)
NPR: 108802925(HACL1)
BBUF: 121002206(HACL1)
BGAR: 122938311(HACL1)
DRE: 406358(hacl1)
SRX: 107743162 107745383
SANH: 107684560 107685543
SGH: 107550801 107596724(hacl1)
CCAR: 109106433(hacl1)
CAUA: 113116276(hacl1)
IPU: 108267388(hacl1)
PHYP: 113542549(hacl1)
SMEO: 124385334(hacl1)
EEE: 113571243(hacl1)
TRU: 101061718(hacl1)
TNG: GSTEN00034133G001
LCO: 104927751(hacl1)
NCC: 104944968(hacl1)
CGOB: 115021252(hacl1)
ELY: 117265388(hacl1)
PLEP: 121945760(hacl1)
SLUC: 116049391(hacl1)
ECRA: 117946488(hacl1)
PFLV: 114557200(hacl1)
GAT: 120811025(hacl1)
PPUG: 119198104(hacl1)
MSAM: 119897754(hacl1)
CUD: 121513857(hacl1)
MZE: 101465513(hacl1)
ONL: 100690060(hacl1)
OAU: 116325395(hacl1)
OLA: 101156193(hacl1)
OML: 112143126(hacl1)
XMA: 102223303(hacl1)
XCO: 114142081(hacl1)
XHE: 116717578(hacl1)
PRET: 103478561(hacl1)
PFOR: 103150389 103150392
PLAI: 106941218 106955235
PMEI: 106922205(hacl1)
GAF: 122831424
CVG: 107103875(hacl1)
CTUL: 119772782(hacl1)
GMU: 124865949(hacl1)
NFU: 107378691(hacl1)
KMR: 108241945(hacl1)
ALIM: 106517744(hacl1)
NWH: 119409454(hacl1)
AOCE: 111588844(hacl1)
CSEM: 103388412(hacl1)
POV: 109633118(hacl1)
SSEN: 122774464(hacl1)
HHIP: 117777286(hacl1)
LCF: 108891319(hacl1)
SDU: 111238787(hacl1)
SLAL: 111652800(hacl1)
XGL: 120784806(hacl1)
HCQ: 109525685(hacl1)
BPEC: 110171659(hacl1)
MALB: 109967232(hacl1)
SASA: 106592607(hacl1) 106596819
OTW: 112225223(hacl1)
OMY: 110505030(hacl1) 110515045 118936608
OGO: 124019490 124023920
SALP: 111971452(hacl1)
SNH: 120038022 120040290
ELS: 105018820(hacl1)
SFM: 108927371(hacl1)
PKI: 111839032(hacl1)
AANG: 118212187(hacl1)
LOC: 102687234(hacl1)
PSPA: 121315127(hacl1)
ARUT: 117394717(hacl1)
LCM: 102362798(HACL1)
CMK: 103181261(hacl1)
RTP: 109933345(hacl1)
BFO: 118407296
BBEL: 109468963
CIN: 100178631
SCLV: 120341709
SPU: 586415
APLC: 110973383
SKO: 100370616(HACL1)
DME: Dmel_CG11208(Hacl)
DER: 6547754
DSE: 6610038
DSI: Dsimw501_GD25296(Dsim_GD25296)
DYA: Dyak_GE13819
DAN: 6496000
DSR: 110183103
DPO: Dpse_GA10842
DPE: 6600980
DMN: 108160101 117192789
DWI: 6640896
DGR: 6568724
DMO: Dmoj_GI18529
DAZ: 108616686
DNV: 108654972
DHE: 111594678
DVI: 6625321
CCAT: 101448871
BOD: 106624777
MDE: 101901094
SCAC: 106096256
LCQ: 111684644
AGA: AgaP_AGAP010368
ACOZ: 120956444
AARA: 120902070
AAG: 5565030 5572369
AALB: 109398509
CQU: CpipJ_CPIJ014172 CpipJ_CPIJ017705
CPII: 120417246
CNS: 116340071
AME: 552738
ACER: 107995125
ALAB: 122717988
BIM: 100744016
BBIF: 117207092
BVK: 117237409
BVAN: 117160978
BTER: 100644973
BPYO: 122572768
CCAL: 108629887
OBB: 114878797
MGEN: 117229721
NMEA: 116434819
CGIG: 122404927
SOC: 105194976
MPHA: 105836739
AEC: 105154992
ACEP: 105626893
PBAR: 105426340
VEM: 105566829
HST: 105189078
DQU: 106750369
CFO: 105255983
FEX: 115243773
LHU: 105669841
PGC: 109857866
OBO: 105282994
PCF: 106784580
PFUC: 122516260
VPS: 122627482
NVI: 100123260
CSOL: 105361881
TPRE: 106658765
MDL: 103573582
CGLO: 123270220
FAS: 105272077
DAM: 107038575
AGIF: 122855352
CCIN: 107263840
TCA: 664029
DPA: 109536893
SOY: 115884215
ATD: 109596968
CSET: 123317367
AGB: 108903720
LDC: 111511260
NVL: 108560626
APLN: 108742675
PPYR: 116180506
OTU: 111426368
BMOR: 692884
BMAN: 114245011
MSEX: 115455821
DPL: KGM_216038
BANY: 112045363
PMAC: 106707335 106712100
PPOT: 106110820
PXU: 106126854
PRAP: 110998130
ZCE: 119839860
HAW: 110380221
TNL: 113492403
OFU: 114354508
PXY: 105381789 105388089 105388092
API: 100165444
DNX: 107163705
AGS: 114132285
RMD: 113551822
BTAB: 109042181
DCI: 103515657
CLEC: 106674218
HHAL: 106686454
NLU: 111047026
PHU: Phum_PHUM529400
FOC: 113211311
ZNE: 110831053
CSEC: 111868986
FCD: 110848423
DPX: DAPPUDRAFT_253560 DAPPUDRAFT_306512
DMK: 116930937
PVM: 113809547 113829823
PJA: 122259379
HAME: 121861697
PCLA: 123761322
PTRU: 123499102
EAF: 111696878
DSV: 119442762
RSAN: 119387927 119387928
RMP: 119188213
VDE: 111248283
VJA: 111271184
CSCU: 111629264
PTEP: 107455176
SDM: 118180287 118202574
CEL: CELE_B0334.3(B0334.3)
CBR: CBG_02874
NAI: NECAME_12882
LGI: LOTGIDRAFT_207618
PCAN: 112564745
BGT: 106078649
GAE: 121382197
HRF: 124141592 124141617
HRJ: 124281687
CRG: 105325181
MYI: 110449719
PMAX: 117343704
MMER: 123531889
OBI: 106872707
OSN: 115212767
LAK: 106162962 106178375
NVE: 5509052
EPA: 110232378 110241064 110251089
ATEN: 116307823
ADF: 107339514 107349360
AMIL: 114965786
PDAM: 113664752
SPIS: 111333468
DGT: 114536270
AQU: 100641768
ATH: AT5G17380
ALY: 9309892
CRB: 17882398
CSAT: 104706028 104735774 104770038
EUS: EUTSA_v10013104mg
BRP: 103833165
BNA: 106383786 106445967 106445973
BOE: 106318756
RSZ: 108817706
THJ: 104820853
CPAP: 110807471
CIT: 102621022
CIC: CICLE_v10011371mg CICLE_v10011616mg
PVY: 116111220
MINC: 123197953 123225748
TCC: 18601969
GRA: 105803616
GHI: 107912763 107912909
GAB: 108453368
DZI: 111305773 111314659
EGR: 104449677
GMX: 100778257 100781584
GSJ: 114384324 114397553
PVU: PHAVU_008G243200g
VRA: 106765165
VAR: 108338133
VUN: 114195378
CCAJ: 109796119
APRC: 113847006
MTR: MTR_6g004200
CAM: 101501749
LJA: Lj2g3v0919440.1(Lj2g3v0919440.1)
ADU: 107489982
AIP: 107644561
AHF: 112751450 112801899
LANG: 109347251
FVE: 101306541
RCN: 112195257
PPER: 18784460
PMUM: 103328654
PAVI: 110764468
PDUL: 117621238
MDM: 103405762 103421889
PXB: 103936009 103948272
MNT: 21409733
CSV: 101218890
CMO: 103487508
BHJ: 120082736
MCHA: 111024979
CMAX: 111492838
CMOS: 111452318 111457768
CPEP: 111788701 111790346
RCU: 8265016
JCU: 105638783
HBR: 110659002
MESC: 110631370
POP: 7477453
PEU: 105133039
PALZ: 118055950
JRE: 108988141
QSU: 112028189
QLO: 115975759
TWL: 119995081 119998680
VVI: 100232933(OXC)
VRI: 117930698
SLY: 101264774
SPEN: 107011723
SOT: 102604551
CANN: 107860091
NTA: 107781829 107825460
NSY: 104211662
NTO: 104096832
NAU: 109243823
INI: 109154528
ITR: 116016327
SIND: 105176140
OEU: 111401032
EGT: 105961104
SSPL: 121746641 121750258 121779556 121783055
HAN: 110868206
ECAD: 122603179
LSV: 111903096
CCAV: 112501516
DCR: 108196317
CSIN: 114315157
BVG: 104906480
SOE: 110801650
CQI: 110703946 110720354
NNU: 104613342
MING: 122068615
TSS: 122645871 122650155
PSOM: 113282348 113341018 113355684
NCOL: 116248481
OSA: 4324491
DOSA: Os01t0505400-01(Os01g0505400)
OBR: 102721129
BDI: 100829473
ATS: 109738743
TDC: 119270248 119278317
TAES: 123063247 123072164 123080437
SBI: 8056536
ZMA: 100191888
SITA: 101779082
SVS: 117857218
PVIR: 120672655 120705520
PHAI: 112895441
PDA: 103705628 103715163
EGU: 105047067 105047158
MUS: 103992223
DCT: 110114604 114579507
PEQ: 110035637
AOF: 109829098
ATR: 18428183
SMO: SELMODRAFT_178690 SELMODRAFT_179619
PPP: 112279961
CSL: COCSUDRAFT_18867
APRO: F751_4156
GSL: Gasu_12440
SCE: YEL020C(PXP1)
AGO: AGOS_AEL055C
ERC: Ecym_1360
LTH: KLTH0H14102g
VPO: Kpol_1061p50
ZRO: ZYRO0E03454g
CGR: CAGL0M03245g
NCS: NCAS_0G03960(NCAS0G03960)
NDI: NDAI_0G00520(NDAI0G00520)
TPF: TPHA_0B03940(TPHA0B03940)
TBL: TBLA_0C01560(TBLA0C01560)
TDL: TDEL_0B05520(TDEL0B05520)
TGB: HG536_0G03620
KAF: KAFR_0H02890(KAFR0H02890)
ZMK: HG535_0B03790
PPA: PAS_chr1-1_0394
YLI: YALI0E07315g
SLB: AWJ20_180
SSL: SS1G_07774
BFU: BCIN_15g01860
MBE: MBM_05575
PSCO: LY89DRAFT_234715
GLZ: GLAREA_03030
ANI: AN1516.2
AFM: AFUA_8G05230
ACT: ACLA_058670
NFI: NFIA_097990
AOR: AO090005000624
ANG: ANI_1_1960144(An16g07490)
AFV: AFLA_003432
ALUC: AKAW2_70591S
ACHE: ACHE_60592S
APUU: APUU_21693S
CIM: CIMG_04305
CPW: CPC735_074190
URE: UREG_01556
PBL: PAAG_11932(PAAG_04811)
PBN: PADG_11446(PADG_02553)
ABE: ARB_06558
TVE: TRV_04424
AJE: HCAG_02805
BGH: BDBG_04203
ZTR: MYCGRDRAFT_100226
PFJ: MYCFIDRAFT_158085
NPA: UCRNP2_5114
TML: GSTUM_00003438001
SPO: SPBC725.04
DDI: DDB_G0292402
DPP: DICPUDRAFT_156484
DFA: DFA_00674
ACAN: ACA1_369090
TET: TTHERM_00503839
PTM: GSPATT00010458001
METR: BSY238_2193
TBH: Tbon_01510
 » show all
Reference
1  [PMID:10468558]
  Authors
Foulon V, Antonenkov VD, Croes K, Waelkens E, Mannaerts GP, Van Veldhoven PP, Casteels M
  Title
Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids.
  Journal
Proc Natl Acad Sci U S A 96:10039-44 (1999)
DOI:10.1073/pnas.96.18.10039
  Sequence
[hsa:26061]
Reference
2  [PMID:15644336]
  Authors
Foulon V, Sniekers M, Huysmans E, Asselberghs S, Mahieu V, Mannaerts GP, Van Veldhoven PP, Casteels M.
  Title
Breakdown of 2-hydroxylated straight chain fatty acids via peroxisomal 2-hydroxyphytanoyl-CoA lyase: a revised pathway for the alpha-oxidation of straight chain fatty acids.
  Journal
J Biol Chem 280:9802-12 (2005)
DOI:10.1074/jbc.M413362200
Reference
3  [PMID:17956236]
  Authors
Casteels M, Sniekers M, Fraccascia P, Mannaerts GP, Van Veldhoven PP
  Title
The role of 2-hydroxyacyl-CoA lyase, a thiamin pyrophosphate-dependent enzyme, in the peroxisomal metabolism of 3-methyl-branched fatty acids and 2-hydroxy straight-chain fatty acids.
  Journal
Biochem Soc Trans 35:876-80 (2007)
DOI:10.1042/BST0350876
Other DBs
ExplorEnz - The Enzyme Database: 4.1.2.63
IUBMB Enzyme Nomenclature: 4.1.2.63
ExPASy - ENZYME nomenclature database: 4.1.2.63
BRENDA, the Enzyme Database: 4.1.2.63

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Chemical substance (5)   
   KEGG COMPOUND (5)   
Chemical reaction (2)   
   KEGG REACTION (2)   
Gene (630)   
   KEGG ORTHOLOGY (1)   
   KEGG GENES (629)   
Protein sequence (8)   
   UniProt (4)   
   SWISS-PROT (4)   
All databases (645)   

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